Optimising liver screening in patients with a Fontan circulation: a cost-effective approach to detection of Fontan associated liver disease which enhances patient care

Abstract Introduction Patients with Fontan circulation, require screening for liver cirrhosis and malignancy, to which they are susceptible with time. Surveillance biopsies reveal nearly all adult Fontan patients develop asymptomatic fibrosis, and a majority have abnormal liver function tests (LFTs). This presents significant financial healthcare burden and psychological stress with testing for patients. Objective We aim to present "real-world" liver screening data, from a leading Adult Congenital Heart Disease (ACHD) centre, to propose a cost-reducing testing regimen, which delivers effective screening, while taking account of the challenges of current guidance, patient compliance, and clinical practice. Methods We analysed liver screening data for Fontan patients at a leading ACHD centre, focusing on biochemical tests (bilirubin, ALT, GGT) and imaging. We evaluated the 10-year financial impact of current practices, versus a hypothetical model based on age and risk factors, alongside biannual and annual screening strategies. Tariffs for biochemical test were provided by the local pathology service and we used NHS HRG tariffs for imaging and phlebotomy. Results We included data from 114 patients with a Fontan circulation (median age 32 range 19-56, 42% females). Eighteen patients developed liver cirrhosis diagnosed on imaging (median age 32, range 22-48). None developed hepatocellular carcinoma. A lack of clear and agreed local screening guidelines (over years) resulted in vastly different investigations for FALD, with inconsistent time intervals for testing. Bilirubin was elevated in 59% (55% in age 18-29), ALT was elevated in 41% (31% in age 18-29), GGT was elevated in 83% (80% in age 18-29). We found 3 cases of cirrhosis in the age group 18-29, all with hypoplastic left heart; 4 cases of cirrhosis in age group 30-34 - all had significant comorbidities; 33% had normal bilirubin, LFT and GGT, potentially not requiring further screening until the age of 35. Eleven patients (22%) age 35+ had confirmed cirrhosis (Figure 1). Performing full FALD screening with biochemistry and liver ultrasound every 12-24 months would increase the cost of screening three- to six-fold. We propose rationalising liver screening by age and underlying risk (Figure 2) to minimise the economic burden and equally the psychological impact of frequent testing on patients. Conclusions Our analysis underscores the necessity for an optimised liver screening protocol in Fontan patients, balancing diagnostic accuracy with cost-efficiency. By integrating this data, we have identified a pathway to streamline screening, tailoring it to individual risk profiles and age groups, thereby reducing unnecessary tests, lowering costs and reducing the impact of testing on patients. This approach not only fosters better patient care, but also provides a framework for developing clearer, cost-effective screening guidelines, to monitor liver health, in these complex CHD patients.